Bone and Soft Tissue Sarcomas - NCIN

Bone and Soft Tissue Sarcomas

UK Incidence and Survival: 1996 to 2010

November 2013 Version 2.0

Author: MF/GL/ND Last updated: 22/10/13 C:\Users\Trish.watts\AppData\Local\Microsoft\Windows\Temporary Internet Files\Content.Outlook\8OY2AKPG\BoneAndSTSInTheUK v2 1.docx Version: 2.1

Copyright @ PHE Knowledge & Intelligence Team (West Midlands) 2013

Authors

Mr Matthew Francis Cancer Analysis Development Manager, Public Health England Knowledge & Intelligence Team (West Midlands)

Dr Nicola Dennis Sarcoma Analyst, Public Health England Knowledge & Intelligence Team (West Midlands)

Ms Jackie Charman Cancer Data Development Analyst

Public Health England Knowledge & Intelligence Team (West Midlands)

Dr Gill Lawrence Breast and Sarcoma Cancer Specialist Analytical Advisor, Public Health England Knowledge & Intelligence Team (West Midlands)

Professor Rob Grimer Consultant Orthopaedic Oncologist

The Royal Orthopaedic Hospital NHS Foundation Trust

For any enquiries regarding the information in this report please contact:

Mr Matthew Francis Public Health England Knowledge & Intelligence Team (West Midlands)

5 St Philip’s Place Birmingham B3 2PW

Tel: 0121 214 9123 E-mail: [email protected]

Version 2.0

This is an updated version of the UK Incidence and Survival 1996 to 2010 report published in July 2013. Alterations made to the first version

- Sarcomas of the skin diagnosed in Scotland are included - Skin sarcomas are excluded from survival rates in all UK countries

Author: MF/ND/GL Last updated: 22/10/13 C:\Users\Trish.watts\AppData\Local\Microsoft\Windows\Temporary Internet Files\Content.Outlook\8OY2AKPG\BoneAndSTSInTheUK v2 1.docx Version: 2.1

CONTENTS

EXECUTIVE SUMMARY .................................................................................................................. 1

1.0 INTRODUCTION .................................................................................................................. 2

2.0 METHODS ............................................................................................................................ 2

3.0 DATA QUALITY ................................................................................................................... 3

4.0 INCIDENCE .......................................................................................................................... 3

4.1 Variation in Bone and Soft Tissue Sarcoma Incidence Rates with Sex ........................ 4 4.2 Variation in Bone and Soft Tissue Sarcoma Incidence Rates with Age ........................ 4 4.3 Variation in Bone and Soft Tissue Sarcoma Incidence Rates with UK Country ........... 5 4.4 Variation in Bone and Soft Tissue Sarcoma Histological Diagnoses with UK Country6 4.5 Variation in Bone and Soft Tissue Sarcoma Anatomical Site with UK Country ........... 6

5.0 SURVIVAL............................................................................................................................ 7 6.0 CONCLUSIONS ................................................................................................................... 9 APPENDIX A COMPREHENSIVE LIST OF SARCOMA MORPHOLOGY CODES…………………11 APPENDIX B ICD-10 SITE CODES INCLUDED WITHIN EACH ANATOMICAL CANCER SITE..14


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EXECUTIVE SUMMARY

Primary bone and soft tissue sarcomas are an exceptionally rare form of cancer, collectively accounting for only 1% of all malignancies diagnosed. This report fills a void in publicly available data describing the incidence and survival of patients diagnosed with primary bone or soft tissue sarcoma in the UK. The report considers overall UK incidence and survival rates, and examines variations amongst the four UK countries. Differences between UK countries in the most common histologies and anatomical sites are also discussed. In 2010 there were 531 new diagnoses of bone sarcoma and 3,298 new diagnoses of soft tissue sarcoma in the UK. The age-standardised incidence of bone sarcoma remained constant at around 7.9 per million between 1996 and 2010. Soft tissue sarcoma incidence rates increased significantly from 39 per million to 45 per million during the same time period. This increase may reflect improved diagnostic techniques and reporting rather than a true increase in incidence. The incidence of soft tissue sarcomas increased significantly with increasing age. The age specific incidence rate was highest in males aged 85 years and over where it reached 230 per million and exceeded the rate for females by a ratio of 1.9:1. Age specific incidence rates for soft tissue sarcomas in females aged 45 to 59 years were slightly higher than those in males, due to the incidence of gynaecological sarcomas. Age specific incidence rates for bone sarcoma were bi-modal, with peaks in incidence observed in teenagers and adolescents, as well as the elderly. Bone sarcoma age specific incidence rates were significantly higher in males than females over 15 years of age. Rates in males exceeded those of females by a ratio of 1.7:1 in those aged 15 to 19 years and by a ratio of 1.6:1 in those aged 55 years and over. The most common site for both bone and soft tissue sarcomas was within the extremities (23% of soft tissue sarcomas and 52% of bone sarcomas). The proportion of Kaposi’s sarcoma was significantly higher in England due to high levels of HIV-linked Kaposi’s sarcoma in the Thames region. Scotland and Wales had a significantly higher proportion of soft tissue sarcomas arising within the gastro-intestinal tract. Owing to the coding practice used within the Northern Ireland Cancer Registry, Northern Ireland had a significantly higher proportion of tumours diagnosed within the thorax and trunk and a significantly lower proportion within the retroperitoneum. This coding practice is the same that is used for retroperitoneal sarcomas by some of the sarcoma specialist centres in England. A national consensus relating to the coding of retroperitoneal sarcomas needs to be agreed if meaningful data on the management of these tumours is to be made available to support centralised specialised commissioning in England. Leiomyosarcoma (22%) and liposarcoma (12%) were the most common soft tissue sarcoma sub-types diagnosed in the UK. Dermatofibrosarcomas accounted for 5% of sarcomas in England, 4% in Wales, 7% in Northern Ireland and 3.5% in Scotland. Northern Ireland had a higher proportion of rhabdomyosarcomas, which could be explained by the younger population age structure within Northern Ireland, and a significantly lower proportion of myxofibrosarcomas (<1%) compared to the UK average (3%). The latter could be a reflection of current pathological reporting within Northern Ireland and requires further investigation. There were significant improvements in soft tissue sarcoma 5-year relative survival rates over the 10-year period studied, with rates increasing from 51% in 1996-2000 to 55% in 2006-2010. From the year 2000 onwards, 5-year relative survival was significantly higher in males than females, by a difference of up to four percentage points. This could be due to the increased incidence of well-differentiated liposarcomas in males. Bone sarcoma 5-year relative survival rates did not change significantly in the 10-year period studied and increased by only two percentage points from 54% in 1996-2000 to 56% in 2001-2005. There were no significant differences between the 5-year relative survival rates for bone sarcomas diagnosed in males and females.


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1.0 INTRODUCTION

Bone and soft tissue sarcomas are a group of rare heterogeneous forms of cancer, which collectively account for approximately 1% of all malignancies diagnosed. Sarcomas represent a challenge to clinicians as they are rare and diagnosis is often delayed. Best practice guidelines advise that they should be treated within centres that specialise in the management of sarcomas. There is a void in the published literature documenting the incidence and survival of bone and soft tissue sarcomas in the UK, as most existing publications are based on either hospital databases or cohort studies. This report presents incidence and survival data for all patients diagnosed with malignant bone or soft tissue sarcomas in the UK between 1996 and 2010. There are over one hundred different morphological sub-types of sarcoma (Appendix A). The most common types of bone sarcoma are osteosarcoma, chondrosarcoma, Ewing’s sarcoma and chordoma. Soft tissue sarcomas develop from soft tissue cells including smooth muscle cells (leiomyosarcomas), fat cells (liposarcomas), fibrous connective tissue (fibrosarcomas), skeletal muscles (rhabdomyosarcomas), synovium (synovial sarcomas), blood vessels (angiosarcomas), breast ducts (phyllodes tumours) and nerves (nerve sheath tumours). Kaposi’s sarcoma is a particular type of sarcoma linked to immune deficiency in patients with HIV infection and is included in this report for completeness. Gastro-intestinal stromal tumours (GISTs) occur in the stomach or small intestine and were frequently coded as leiomyosarcomas in the past. As there are inconsistencies in the coding of GISTs across the United Kingdom (UK), incidence and survival data have not been included as a separate entity in this report, and these tumours have been included in the leiomyosarcoma category. This report presents data for the 5 most common variants of bone sarcoma. The 22 most common variants of soft tissue sarcoma have been grouped into 12 sub-types, and the remaining less common variants are represented in a separate group. For the purpose of reporting incidence and survival, soft tissue sarcomas in the 12 morphological sub-type groups were further combined according to the anatomical diagnostic site groupings in Appendix B. 2.0 METHODS

The Public Health England, Knowledge and Intelligence Team (West Midlands) (WM KIT) is the National Cancer Intelligence Network (NCIN) lead intelligence team in England for bone and soft tissue sarcoma. As such, the WM KIT analyses national data on the incidence, mortality, survival and treatment of bone and soft tissue sarcomas in England. These analyses are usually conducted using the National Cancer Data Repository (NCDR), a compilation of data collected by the eight English regional cancer registration offices that now form the National Cancer Registration Service (NCRS). The current version of the NCDR also includes cancer registry data from Scotland, Wales and Northern Ireland, enabling the analyses of bone and soft tissue sarcomas within the UK. Bone and soft tissue sarcomas are classified by both the tenth revision of the International Classification of Diseases (ICD-10) site code and their morphology. ICD-10 is the standard global system for reporting mortality and morbidity statistics1. Within that system, the prefix ‘C’ locates the code within the ‘neoplasm’, or cancer, sub-group, and the numbers that follow localise the tumour to a specific area of the body. A two number string denotes a general area of the body, while a three number string represents a more specific area; for example, ‘C-03’ denotes a malignant neoplasm of the gum, and ‘C-031’ represents a malignant neoplasm of the lower gum. Bone sarcomas are identified specifically from ICD-10 site codes C40 (malignant neoplasm of bone and articular cartilage of limbs) and C41 (malignant neoplasm of bone and articular cartilage of other and unspecified sites). Sarcomas arising within all anatomical cancer sites outside of the bone are soft tissue sarcomas. All malignant sarcomas (excluding sarcomas of the brain, ICD-10 codes C70, C71 and C72) are included in the analyses in this report. The current version of the NCDR includes all malignancies


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diagnosed in England in the period 1990 to 2010. However, data for Northern Ireland do not commence until 1993. Potential coding issues with regard to the soft tissue sarcomas recorded within the NCDR also exist, particularly those registered in the early 1990’s. Therefore, the analyses in this report have been restricted to incidence and survival rates for bone and soft tissue sarcomas diagnosed in the UK in the 15-year period between 1996 and 2010. Confidence intervals around incidence rates were calculated using the gamma method. Relative survival is defined as the observed survival in the patient group divided by the expected survival of the general population, matched by age, sex, and calendar year. Relative survival was calculated in Stata (v.11) using the strs programme which calculates relative survival estimates using the Ederer II method2. National life tables were obtained from the Cancer Research UK Cancer Survival Group at the London School of Hygiene and Tropical Medicine. Five-year relative survival was calculated using 5-year rolling averages. 3.0 DATA QUALITY Historically, to construct the NCDR, the eight NCRS cancer registration offices in England submitted the details for all malignant tumours recorded within their local cancer registration database, regardless of whether or not the patient resided within the corresponding cancer registry’s catchment area. Thus, cancer registries submitted both intra- and extra-regional tumour details. In the most recent iteration of the NCDR covering tumour diagnoses between 1990 and 2010, the cancer registration offices were asked to submit only their intra-regional cases in order to avoid duplication of tumour details. An investigation of the NCDR was undertaken to identify the number of potential duplicate sarcomas recorded in the latest iteration of the NCDR. Three hundred and fourteen (approximately 14 to 15 per year) sarcomas appear to have been recorded more than once either within the same registry, or across two or more cancer registries. The number of potential duplicate tumours recorded annually is insignificant in comparison to the 2,800 sarcomas diagnosed annually. However, the greatest issue appears to relate to 1999 where the tumour details for around 50 sarcomas appear to have been duplicated. There are also “pockets” of the country where the United Kingdom and Ireland Association of Cancer Registries (UKIACR) postcode directory specifies that the postcode belongs to a particular cancer registry, but where two (or more) registration offices have submitted cancer registration details for the same sarcoma.

Table 1: Potential duplication of tumours recorded within the NCDR (England: 1990-2010)

Table 1 summarises the duplicate sarcomas identified. Around 70% of the duplicate registrations were within the same cancer registration office (i.e. a single cancer registration office has registered the same tumour more than once). The remaining duplicates were cross-regional (i.e. more than one registration office has registered the same tumour). The potential duplicates identified in Table 1 are to be queried directly with the appropriate cancer registration office.

4.0 INCIDENCE

In the UK between 1996 and 2010, 43,590 patients were diagnosed with a soft tissue sarcoma (3,298 in 2010) and 7,229 patients with a bone sarcoma (531 in 2010). In 2010, the soft tissue

Cancer Registration

Office

East

Midlands

Eastern

Office

North

West

Northern &

YorkshireSouth East

South

WestThames

West

Midlands

Grand

Total

East Midlands 23 2 25

Eastern Office 2 35 2 39

North West 2 1 11 2 1 9 8 34

Northern & Yorkshire 17 17

South East 2 1 5 5 13

South West 8 8 4 70 19 109

Thames 5 7 3 53 68

West Midlands 2 1 1 1 4 9

Grand Total 44 52 11 31 6 80 86 4 314


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sarcoma age-standardised incidence rate (ASR) was 45 per million, and the bone sarcoma age-standardised incidence rate (ASR) was 8.2 per million (Figures 1a and 1b). These observed incidence rates are similar to the incidence rates for bone and soft tissue sarcoma reported by other cancer registries outside the UK3,4,5.

Soft tissue sarcoma age-standardised incidence rates have increased gradually, but significantly, from 39 per million in 1996 to 45 per million in 2010. Bone sarcoma incidence rates remained constant between 1996 and 2006 at 7.4 per million, but increased significantly to 9.2 per million in 2007. Between 2009 and 2010, bone sarcoma incidence rates decreased slightly to 8.2 per million, and are not significantly higher than the incidence rate observed between 1996 and 2006. The higher bone sarcoma incidence rates in 2007 and 2008 were confined to England and were predominantly in the Thames Cancer Registry catchment area.

4.1 Variation in Bone and Soft Tissue Sarcoma Incidence Rates with Sex

Male soft tissue sarcoma age-standardised incidence rates were higher than those in females throughout the 15-year period studied, but the rates were significantly higher only in 1996, 2008 and 2010 (Figure 1a). Bone sarcoma age-standardised rates in males were also consistently higher than those of females, and this difference was statistically significant between 2004 and 2010 (Figure 1b).

4.2 Variation in Bone and Soft Tissue Sarcoma Incidence Rates with Age

The incidence of soft tissue sarcomas increased significantly with increasing age (Figure 2a). The age specific incidence rate (ASIR) was highest in males aged 85 years and over where it reached 230 per million (23 per 100,000) and exceeded the rate for females by a ratio of 1.9:1. Age specific incidence rates for soft tissue sarcomas in females aged 45 to 59 years were slightly higher than those in males due to the incidence of gynaecological sarcomas.

Age specific incidence rates (ASIR) for bone sarcoma were bi-modal, with peaks in incidence observed in teenagers and adolescents, as well as the elderly (Figure 2b). Bone sarcoma age specific incidence rates were significantly higher in males than in females over 15 years of age. Rates in males exceeded those of females by a ratio of 1.7:1 in those aged 15 to 19 years and by a ratio of 1.6:1 in those aged 55 years and over.

Figure 1a: Five-year rolling soft tissue sarcoma age-standardised incidence rates

(UK: 1996-2010)

Figure 1b: Five-year rolling bone sarcoma age-standardised incidence rates

(UK: 1996-2010)

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4.3 Variation in Bone and Soft Tissue Sarcoma Incidence Rates with UK Country

On average, 2,740 soft tissue sarcomas were diagnosed annually in England, 270 in Scotland, 180 in Wales and 80 in Northern Ireland. In 2010, 443 bone sarcomas were diagnosed in England, 48 in Scotland, 23 in Wales and 17 in Northern Ireland (Table 2).

Table 2: United Kingdom populations and bone and soft tissue sarcoma incidence (2010)

CI = 95% confidence intervals CI = 95% confidence intervals

Figure 3a: Five-year rolling soft tissue sarcoma age-standardised incidence rates for each country

(UK: 1996-2010)

Figure 3b: Five-year rolling bone sarcoma age-standardised incidence rates for each country

(UK: 1996-2010)

Figure 2a: Soft tissue sarcoma age specific incidence rates (United Kingdom: 1996-2010)

Figure 2b: Bone sarcoma age specific incidence rates (United Kingdom: 1996-2010)

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England 52,234,045 2,740 44.9 (43.0 - 47.0) 443 8.2 (8.0 - 9.0)

Scotland 5,222,100 274 43.4 (38.3 - 49.4) 48 8.9 (6.6 - 12.2)

Wales 3,006,430 201 53.4 (46.0 - 62.4) 23 7.1 (4.5 - 11.5)

Northern Ireland 1,799,392 83 46.0 (34.0 - 54.0) 17 9.6 (5.8 - 16.0)

United Kingdom 62,261,967 3,298 45.1 (43.5 - 46.7) 531 8.2 (7.6 - 9.0)

Soft Tissue Bone

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Soft tissue sarcoma age-standardised incidence rates did not differ significantly amongst the four UK countries (Figure 3a). Bone sarcoma age-standardised incidence rates did not differ significantly amongst the four UK countries, although oscillations within the Northern Ireland rates could be seen (Figure 3b). This is due to the relatively low number of tumours diagnosed in the much smaller population of Northern Ireland (Table 2).

4.4 Variation in Bone and Soft Tissue Sarcoma Histological Diagnoses with UK Country

Leiomyosarcoma (including GISTs) (22%) and liposarcoma (12%) were the most common specific types of soft tissue sarcoma diagnosed in the UK. A further 20% of sarcomas were diagnosed as “sarcoma; not otherwise specified (NOS)” which could be indicative of either imprecise registration by cancer registries or the inability to make a specific diagnosis. A significantly lower proportion of sarcomas in England were diagnosed as sarcoma NOS compared to the other three UK countries (Figure 4a). There were also differences in the proportion of dermatofibrosarcomas recorded in each of the four UK countries. These tumours accounted for 5% of sarcomas in England, 4% in Wales, 7% in Northern Ireland and 3.5% in Scotland. Northern Ireland had a higher proportion of rhabdomyosarcomas (RMS), which could be explained by the younger population age structure within Northern Ireland. Northern Ireland also had a significantly lower proportion of myxofibrosarcomas (<1%) compared to the UK average (3%). This could be a reflection of current pathological reporting within Northern Ireland and requires further investigation.

Osteosarcoma is frequently cited as the most commonly diagnosed morphological sub-type variant arising in the bones6. However, in more recent years, chondrosarcoma appeared to be more commonly diagnosed than osteosarcoma. The proportion of bone sarcomas diagnosed as Ewing’s sarcoma was significantly higher in Northern Ireland and Scotland, to the extent that Ewing’s sarcomas were more common than osteosarcomas in Northern Ireland. There were no cases of giant cell tumours of the bone recorded in Northern Ireland. This could be a potential data quality issue and requires further investigation (Figure 4b).

4.5 Variation in Bone and Soft Tissue Sarcoma Anatomical Site with UK Country

The most common anatomical cancer sites for soft tissue sarcomas to arise were the extremities (23%), the trunk and thorax (including the intra-abdominal cavity [14%]) and the female genital

Figure 4a: Proportion of soft tissue sarcoma histological diagnoses in each country

(UK: 1996-2010)

Figure 4b: Proportion of bone sarcoma histological diagnoses in each country

(UK: 1996-2010)

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tract (11%) (Figure 5a). England had fewer tumours diagnosed within the head and neck region (5%), but a significantly higher proportion of Kaposi’s sarcoma (5%). This is due to the high levels of HIV-linked Kaposi’s sarcoma diagnosed within the population covered by the London cancer registration office.

Around 5% of sarcomas arose within the retroperitoneum. This is considerably lower than the 10-20% reported for individual hospital databases. Northern Ireland had a significantly higher proportion of tumours diagnosed within the thorax and trunk (14%), and a significantly lower proportion within the retroperitoneum (<1%). This is due to the tumour coding practice within the Northern Ireland Cancer Registry; retroperitoneal sarcomas are recorded as abdominal tumours (ICD-10 code C494) and consequently included within the thorax and trunk. The coding of retroperitoneal sarcomas in Northern Ireland is the same as the coding used for retroperitoneal sarcomas by some of the sarcoma specialist centres in England. A national consensus relating to the coding of retroperitoneal sarcomas needs to be agreed if meaningful data on the management of these tumours is to be made available to support centralised specialised commissioning in England. Scotland and Wales had a significantly higher proportion of soft tissue sarcomas arising within the gastro-intestinal tract compared to England and Northern Ireland. Northern Ireland had significantly fewer soft tissue sarcomas recorded as “other” or “unspecified” cancer sites (C498 and C499). Around 50% of bone sarcomas were diagnosed in the bones of the extremities, 16% within the pelvic bones and 10% within the bones of the skull and face (Figure 5b). There were no significant differences amongst the four UK countries. Northern Ireland had a higher proportion of bone sarcomas within “other” or unspecified bones (18%) and slightly fewer arising within the pelvic bones and extremities. This is a reflection of pathological reporting in Northern Ireland, where more accurate diagnostic information is required. 5.0 SURVIVAL

For the purpose of calculating 5-year relative survival rates, sarcomas arising in the skin were excluded. Skin sarcomas are predominantly dermatofibrosarcomas for which five-year relative survival rates approach 100%. Overall soft tissue sarcoma 5-year relative survival rates increased significantly between 1996-2000 and 2001-2005 from 50% to 54%. Soft tissue sarcoma 5-year relative survival rates improved from 51% to 55% in males and from 49% to 52% in females (Figure 6a). During the first half of the time period examined there were no significant differences between male and female 5-year relative survival rates for soft tissue sarcoma. However, from the year 2000 onwards, 5-year

Figure 5a: Proportion of soft tissue sarcomas arising at each cancer site in each country

(UK: 1996-2010)

Figure 5b: Proportion of bone sarcomas arising at each cancer site in each country

(UK: 1996-2010)

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relative survival was significantly higher in males than females by a difference of up to four percentage points (Figure 6a). This could result from the increased incidence of well-differentiated liposarcomas in males which have relative survival rates as high as 100% depending on the anatomical cancer site of diagnosis. Bone sarcoma 5-year relative survival rates did not change significantly in the 10-year period studied, and increased by only two percentage points from 54% in 1996-2000 to 56% in 2001-2005. There were no significant differences between the 5-year relative survival rates for bone sarcomas diagnosed in males and females (Figure 6b).

CI = 95% confidence intervals CI = 95% confidence intervals

Throughout the 10-year period studied, soft tissue sarcoma 5-year relative survival rates were always slightly higher in England and Northern Ireland than in Wales and Scotland, but these

Figure 7a: Soft tissue sarcoma (excluding skin) 5-year rolling 5-year relative survival rates

(UK: 1996-2005)

Figure 7b: Bone sarcoma 5-year rolling 5-year relative survival rates

(UK: 1996-2005)

Figure 6a: Soft tissue sarcoma (excluding skin) 5-year rolling 5-year relative survival rates

in males and females (UK: 1996-2010)

Figure 6b: Bone sarcoma 5-year rolling 5-year relative survival rates in males and females

(UK: 1996-2010)

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differences were not statistically significant (Figure 7a). Bone sarcoma survival rates in Northern Ireland appeared to be higher than those of the other three UK countries (Figure 7b), but this difference was not significant owing to the very small numbers diagnosed. There are no comparative international studies with which to compare the overall soft tissue sarcoma 5-year relative survival rates found in this study. Survival rates for each histological sub-type and cancer site, are available for bone and soft tissue sarcomas diagnosed in England between 1985 and 20097,8. Bone sarcoma overall 5-year relative survival rates for the UK are considerably lower than the 66% 5-year relative survival rate reported within the “Surveillance Epidemiology and End Results” (SEER) programme (based on 2003-2009)9.

6.0 CONCLUSIONS

Bone and soft tissue sarcomas are exceptionally rare. This is the first time that incidence and survival statistics for the UK have been published. Soft tissue sarcoma and bone sarcoma age-standardised incidence rates were 45 per million and 8 per million respectively in the period 2006-2010. There were no statistically significant differences in the overall incidence of bone or soft tissue sarcomas or 5-year relative survival rates amongst the four UK countries. There were some differences between UK countries in the types of sarcomas diagnosed and in the underlying anatomical sites. Northern Ireland had significantly higher rates of bone sarcomas arising in other, or unknown, cancer sites. This highlights an area for further investigation to see whether it reflects the need for more detailed pathology reporting or more specific coding by the Northern Ireland Cancer Registry. Northern Ireland had fewer soft tissue sarcomas arising at unspecified cancer sites, and the proportion of myxofibrosarcomas diagnosed in Northern Ireland was significantly lower than that for the other UK countries. Soft tissue sarcoma 5-year relative survival rates improved significantly between 1996 and 2005, from 51% to 55% for males, and from 49% to 52% for females. Bone sarcoma 5-year relative survival rates remained consistent at around 54%. Overall bone and soft tissue sarcoma 5-year relative survival rates were almost identical amongst the four UK countries. Bone sarcoma survival rates in Northern Ireland appeared higher than those in the three other UK countries, but these differences were not statistically significant owing to the very small numbers diagnosed. A major limiting factor of the data presented in this report is that the stage of diagnosis for sarcomas diagnosed in the UK was incomplete. It was therefore not possible to adjust the survival analyses to reflect the underlying stage at diagnosis. Staging data will be collected in future in England as part of the new Cancer Outcomes and Services Dataset (COSD) which is now a mandatory requirement for all hospitals treating NHS patients.


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7 Francis et al (2013) Bone sarcoma incidence and survival – Tumours diagnosed between 1985 and 2009 [online], Available: www.ncin.org.uk/view.aspx?rid=1649 [17th April 2013]

8 Francis et al (2013) Soft tissue sarcoma incidence and survival – Tumours diagnosed between 1985 and 2009 [online], Available: www.ncin.org.uk/view.aspx?rid=2062 [17th April 2013]

9 Howlader N, Noone AM, Krapcho M, Garshell J, Neyman N, Altekruse SF, Kosary CL, Yu M, Ruhl J, Tatalovich Z, Cho H, Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975-2010, National Cancer Institute. Bethesda, MD, http://seer.cancer.gov/csr/1975_2010/, based on November 2012 SEER data submission, posted to the SEER web site, 2013.


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APPENDIX A – Comprehensive List of sarcoma Morphology Codes

(WHO 2012. International Classification of Diseases for Oncology, 3rd Edition (ICD-O-3).)

Morphology Description

8710 Glomangiosarcoma: Glomoid sarcoma

8711 Glomus tumour (nad varients), malignant glomus tumour

8713 Glomangiomyoma

8800 Sarcoma, NOS

8801 Spindle cell sarcoma

8802 Giant cell sarcoma (except of bone M9250/3); pleomorphic cell sarcoma

8803 Small cell sarcoma; round cell sarcoma

8804 Epithelioid sarcoma, epithelioid cell sarcoma

8805 Undifferentiated sarcoma

8806 Desmoplastic small round cell tumour

8810 Fibrosarcoma, NOS, sclerosing epitheliod fibrosarcoma

8811 Fibromyxosarcoma

8812 Periosteal fibrosarcoma (C40._, C41._); periosteal sarcoma, NOS (C40._, C41._)

8813 Fascial fibrosarcoma

8814 Infantile fibrosarcoma; congenital fibrosarcoma

8815 Solitary fibrous tumour, NOS

8821 Aggressive fibromatosis, Desmoid tumour NOS

8822 Abdominal fibromatosis (ICDO-2)

8823 Desmoplastic fibroma (ICD-O-2)

8824 Myofibromatosis (ICD-O3)

8825 Inflammatory myofibroblastic tumour, Myofibroblastic tumour, NOS

8830 Fibrous histiocytoma, malignant; fibroxanthoma, malignant

8832 Dermatofibrosarcoma, NOS (C44._); dermatofibrosarcoma protuberans, NOS (C44._)

8833 Pigmented dermatofibrosarcoma protuberans; Bednar tumour

8834 Giant cell fibroblastoma

8835 Plexiform fibrohistiocytic tumour

8836 Angiomatoid fibrous histiocytoma

8840 Myxosarcoma

8841 Angiomyxoma

8842 Ossifying fibromyxoid tumour, atypical

8850 Liposarcoma, NOS; fibroliposarcoma

8851 Liposarcoma, well differentiated; Liposarcoma, differentiated

8852 Myxoid Liposarcoma; myxoliposarcoma

8853 Round cell liposarcoma

8854 Pleomorphic liposarcoma

8855 Mixed liposarcoma

8857 Fibroblastic liposarcoma

8858 Dedifferentiated liposarcoma

8860 Angiomyoliposarcoma

8890 Leiomyosarcoma, NOS

8891 Epithelioid leiomyosarcoma

8894 Angiomyosarcoma

8895 Myosarcoma

8896 Myxoid leiomyosarcoma

8897 Smooth muscle tumour

8898 Metastasizing leiomyosarcoma

8900 Rhabdomyosarcoma, NOS; rhabdosarcoma

8901 Pleomorphic rhabdomyosarcoma

8902 Mixed type rhabdomyosarcoma


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8910 Embryonal rhabdomyosarcoma; sarcoma botryoides; botryoid sarcoma

8912 Spindle cell rhabdomyosarcoma

8920 Alveolar rhabdomyosarcoma

8921 Rhabdomyosarcoma with ganglionic differentiation; Ectomesenchymoma

8930 Endometrial stromal sarcoma (C54.1)

8931 Endometrial stromal sarcoma, low grade

8935 Stromal Sarcoma

8936 Gastrointestinal stromal sarcoma

8963 Rhabdoid sarcoma

8964 Clear cell sarcoma of kidney

8982 Myoepithelioma

8990 Mesenchymoma, malignant; mixed mesenchymal sarcoma

8991 Embryonal sarcoma

9020 Phyllodes tumour, malignant (C50.) Cystosarcoma phyllodes, malignant (C50.)

9040 Synovial sarcoma, NOS; synovioma, NOS; synovioma, malignant

9041 Synovial sarcoma, spindle cell

9042 Synovial sarcoma, epithelioid cell

9043 Synovial sarcoma, biphasic

9044 Clear cell sarcoma (except of kidney M8964/3)

9120 Haemangiosarcoma, Angiosarcoma of soft tissue

9130 Haemangioendothelioma, NOS, Kaposiform haemangioepithelioma

9133 Epithelioid haemangioendothelioma, malignant

9135 Endovascular papillary angioendothelioma

9136 Spindle cell hemangioendothelioma

9140 Kaposi sarcoma; Multiple haemorrhagic sarcoma

9150 Haemangiopericytoma, NOS

9170 Lymphangiosarcoma; lymphangioendothelial sarcoma

9174 Lymphangiomyomatosis

9180 Osteosarcoma, NOS (C40._, C41._)

9181 Chondroblastic osteosarcoma (C40._, C41._)

9182 Fibroblastic osteosarcoma (C40._, C41._); osteofibrosarcoma (C40._, C41._)

9183 Telangiectatic osteosarcoma (C40._, C41._)

9184 Osteosarcoma in Paget's disease of bone (C40._, C41._)

9185 Small cell osteosarcoma (C40._, C41._)

9186 Central osteosarcoma (C40._, C41._);

9187 Intraosseous well differentiated osteosarcoma (C40._, C41._)

9190 juxtacortical osteosarcoma ICD-O-2

9192 Parosteal osteosarcoma (C40._, C41._)

9193 Periosteal osteogenic sarcoma (C40._, C41._)

9194 High grade surface osteosarcoma (C40._, C41._)

9195 Intracortical osteosarcoma (C40._, C41._)

9200 Aggressive osteoblastoma

9210 Osteochondromatosis

9220 Chondrosarcoma

9221 Juxtacortical chondrosarcoma (C40._, C41._)

9230 Chondroblastoma, malignant (C40._, C41._)

9231 Myxoid chondrosarcoma

9240 Mesenchymal chondrosarcoma

9242 Clear cell chondrosarcoma, (C40._, C41._)

9243 Dedifferentiated chondrosarcoma (C40._, C41._)

9250 Giant cell tumour of bone, NOS

9251 Giant cell tumour of soft parts, NOS


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9252 Malignant tenosynovial giant cell tumour (C49._)

9260 Ewing's sarcoma, Ewing's tumour, Extraskeletal Ewing tumour

9261 Adamantinoma of long bones; tibial adamantinoma (C40.2)

9270 Odontogenic tumour

9290 Ameloblastic odontosarcoma: Ameloblastic fibrodentinosarcoma

9310 Ameloblastoma

9330 Ameloblastic fibrosarcoma: Ameloblastic sarcoma: Odontogenic fibrosarcoma

9341 Clear cell odontogenic tumour

9342 Odontogenic carcinosarcoma

9364 Peripheral neuroectodermal tumour; neuroectodermal tumour, NOS

9365 Askin tumour

9370 Chordoma

9371 Chondroid chordoma

9372 Dedifferentiated chordoma

9373 Parachondroma

9473 Primitive neuroectodermal tumour

9540 Malignant peripheral nerve sheath tumour MPNST, NOS

9560 Malignant schwannoma; neurilemoma, malignant

9561 Malignant peripheral nerve sheath tumour with rhabdomyoblastic differentiation

9571 Perineurioma, malignant; Perineural MPNST

9580 Granular cell tumour, malignant; granular cell myoblastoma, malignant

9581 Alveolar soft part sarcoma


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APPENDIX B – ICD-10 Site Codes Included Within Each Anatomical Cancer Site

Bone and Soft Tissue Sarcomas - NCIN

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